Double outlet right ventricle

The double outlet right ventricle ( DORV ) is a group of congenital malformation of the heart in the large arteries and aorta pulmonary artery exclusively from the right ventricle arise. In parallel, there is necessarily always a ventricular septal defect ( VSD), which represents the single output of the left ventricle, and frequently also a pulmonary stenosis. It may be a transposition of the great arteries, which are referred to in this case as a D-or L - malposition present.

Also, an origin of the aorta and pulmonary artery from the left ventricle ( double outlet left ventricle, DOLV ) occurs in rare cases, clinic and therapy of the same DORV.

Formation

Since the malformation is caused by a disturbed rotation of the outflow tract of the heart during embryonic development, it is considered a sign of heterotaxy. In different frequency of heart failure is associated with other malformations such as atrial septal, patent ductus arteriosus or a coarctation of the aorta.

Classification

An important influence on hemodynamics and the clinical expression has the location of the VSD and the presence and severity of pulmonary stenosis.

Anatomically, the borders subaortale form of DORV with pulmonary stenosis of the tetralogy of Fallot only by the displacement of the aorta at least 50% on the side of the right ventricle from.

Clinic

Generally there are different distinct signs of heart failure. In addition, general signs of congenital heart defects such as poor feeding and failure to thrive.

DORV without pulmonary stenosis

About the subaortalem VSD oxygenated, oxygen-rich blood from the lungs flows mainly in the systemic circulation, the venous blood flows mainly the regular route via the pulmonary artery to the lungs. The clinical picture and hemodynamics according largely to the pressure of a non -separating VSD.

The VSD subpulmonalem the arterialized blood flow into the lungs, the venous blood primarily in the body, with the result of cyanosis. The image is similar to a transposition of the great arteries with VSD. Uncorrected it comes in the wake of the pressure load of the lung in pulmonary hypertension.

The clinical features of mixed form depends on the exact location of the VSD.

In the absence of proximity of the VSD to the aorta or pulmonary artery, there is a mixing of arterialized venous blood and. Clinically there is a cyanosis. Patients are often a function of a patent ductus arteriosus ( Duktusabhängiggkeit ).

DORV with pulmonary stenosis ( tetralogy of form)

During a pulmonary stenosis there is largely a picture of a tetralogy of Fallot. When subaortalem without VSD, with all other forms of cyanosis. Depending on the severity of the pulmonary stenosis is possibly a duktus dependent.

The form of this heart defect can be very different, even after which malformation combination exists. In some cases, the clinical picture is hanging in the delineation of Fallot's tetralogy only on the more or less riding aorta. About the VSD venous and arterial blood is mixed. The result is a cyanosis.

Diagnostics

  • Echocardiography as demonstrative non- invasive method
  • EKG ( signs of right heart strain )
  • X, with possibly uncharacteristic signs of increased or decreased pulmonary blood flow
  • Cardiac catheterization usually as a complementary examination for surgical planning ( particularly in a complex malformation )

Therapy

The surgical correction using the heart -lung machine depends on the severity of the heart defect. The aim is to produce a normal or largely of standard approximate anatomy. You can:

  • One-stage correction for "simple types"
  • Closure of the VSD and correction of pulmonary stenosis by extension or implantation of a homograft (human donor valve)
  • The VSD is below the pulmonary valve or below both large arteries and far removed from the vessels in the muscles of the septum is a Rastelli operation for use.
  • Is assigned by the closure of the VSD, the right ventricle of the aorta and the left ventricle of the pulmonary artery, and there is a transposition of the great arteries, one is carried out " arterial switch operation ".
  • At surgery after Damus - Kaye - Stensel the transposition of the great arteries is corrected by the fact that the pulmonary artery is connected laterally to the aorta
  • Rarely the Fontan operation is in very complicated anatomical conditions

Sometimes the correction operation, the system of aorto - pulmonary shunt is preceded (in bright cyanosis) or the system of a Pulmonary banding ( VSD without pulmonary stenosis subpulmonaler ).

Treatment outcomes and prognosis

The correction can be with very good operational results relatively simple. For complex shapes can have problems later. A slight persisting narrowing or leakage of the pulmonary stenosis may become more important and make a later operation necessary. A little further existing VSD has no meaning in general. A narrowing in the outflow tract of the left ventricle ( aortic valve ) usually makes a rapid re-intervention necessary. Since this operation is often near the conduction system, permanent cardiac arrhythmias may occur. General predictions are not possible, given the diversity of this heart defect.

Lifetime check-ups are necessary. Likewise, compliance with endocarditis.

Literature and sources

  • Keck; Hausdorf (eds): Pediatric Cardiology. 5 edition. Urban & Fischer, Munich; Jena 2002, ISBN 3-437-21960- X, pp. 213ff.
  • Thomas Borth- Bruhns, Andrea Eichler: Pediatric Cardiology. 1 edition. Springer, Berlin 2004, ISBN 978-3-540-40616-7, pp. 151ff.
  • S2k guideline Double Outlet Right Ventricle ( DORV ) of the German Society of Pediatric Cardiology ( DGPK ). In: AWMF online (as of 2013)
  • Disease in pediatric
Congenital disorder Situs ambiguus Atrial septal defect Overriding aorta Medical guideline
244699
de